Transcript ID Case Conference 10-10-07

ID Case Conference
April 9, 2008
Gretchen Shaughnessy, MD
Clinical Fellow
Dept of Infectious Diseases
CC: SOB
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46M presented to PCP with SOB and cough
productive of white sputum. No
hemoptysis, cough had been progressive
over the past 3 weeks. He had been on
Biaxin x 10 days without improvement.
He’d also noticed some orthopnea, but
denied any post-nocturnal dyspnea. Over
the past 4-6 weeks he reported a 20lb wt
loss and intermittent night sweats. Admitted
to Outside Hospital for further workup.
HPI
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During admission to Outside Hospital the patient
had a normal echo (no pulm HTN), chest CT with
moderate R pleural effusion and LLL interstitial
process.
Sputum cultures and pleural fluid analysis and
cultures were done.
Bronchoscopy with biopsy done day 6 of
hospitalization, complicated by tension
pneumothorax requiring chest tube placement.
Despite broad specturm antibiotics and chest tube
placement his respiratory status declined from
11/14-11/23. Transferred to UNC MICU.
PMH
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HTN
DM – type II
GERD
Hiatial Hernia
Hyperlipidemia
h/o R shoulder surgery in 2001
CRI – baseline Cr 1.6
Medications
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Allergies – NKDA
Atenolol 100mg daily
Nexium 40mg daily
Glyburide 5mg po BID
Antibiotic History
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11/4-11/14 Biaxin
11/14-11/23 Azithromycin &
Ticaricillin/clavulanate
11/21-11/23 Methylpredisolone 60mg
IV q8h
Social History
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No tob, ETOH, or drugs.
Currently works in construction, former truck driver.
A few weeks ago was sweeping parking lots and
exposed to a lot of dust.
No recent sick contacts.
No recent travel.
Frequently rides in a friend’s van that is used to
transport chickens and rabbits (patient has never
encountered the animals in person)
History of travel to the southwest as a truck driver,
but none in the past 2 years.
Denies HIV risk factors, has never been tested.
Family History
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Mother - DM type II
Father – ETOH induced liver disease
No family history of autoimmune disease
Patient’s mother had Mycobacterial
Tuberculosis 15 years ago. He reports 6
months of treatment for his mother and the
whole family had to get PPDs placed. The
patient’s PPD was positive but he does not
remember getting LTBI treatment.
ROS
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Night sweats and weight loss for 6
weeks
No chest pain, no N/V/D, no BRBPR,
no hematuria or dysuria.
No joint pain or swelling.
No rashes or skin lesions.
Otherwise negative ROS.
Physical Exam
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T 37.7 - HR 110 - RR 36 BP 95/65 – 99% on 100%
NRB
Tachypnec, on 100% NRB
a&ox3, pleasant and
cooperative
mild errythema seen on the
ventral surface of the elbow
on the RUE
soft NT nabs, no HSM
no c/c/e
nl tone, full ROM present
no focal defecits
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EOMI, PERRLA, nonicteric
no e/e on OP
no JVD
no LAD appreciated in
cervical, supraclavicular, or
inguinal regions
II/VI systolic murmur
decreased breath sounds at
the bases. L chest wall is
higher than R, crepitus
present. chest tube present
OSH Labs
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Pleural fluid - exudative process, pH was 8 and the
glucose was reportedly elevated
Micro from bronch- gram stain many WBCs, yeast,
rare GPCs.
aerobic cultures candida albicans only
anaerobic cultures no growth
fungal cultures growing candida albicans only
AFB smear and culture pending at the state lab.
Bronch biopsy – diffuse fibrosis and inflammation,
?UIP
OSH Labs
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Urine microscopy: granular casts,
monomorphic red cells, and yeast with
pseudohyphae
urine microalbumin 100 (11/24), UP/C 4.069
UA 1.010/5.0/1+ protein/3+ blood/7
WBC/148 RBC/4 granular casts/occ bacteria
HgbA1C 8.2
ESR 68
SPEP and UPEP negative
ANA, ENA panel negative
Radiology
Discussion
Labs
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Serum crypto, urine histo, urine
legionella negative
PCP DFA negative from bronch
AFB smears and cultures from bronch,
pleural fluid, all negative
Further Hospital Course
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Patient had slides from lung biopsy sent to
UNC
Pathology showed findings concerning for
acute interstitial fibrosis. AFB, bacterial,
viral, and fungal cultures all negative.
Hypoxia progressed, ARDS, unable to
oxygenate
Patient expired on post-transfer day #10
“A Diagnostic test was
performed…”
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Unfortunately, autopsy. (“Pathologists always get
the diagnosis… just a little late.”)
Late exudative stage of early organizing stage
idiopathic diffuse alveolar damage (acute interstitial
pneumonia).
– Severe bilateral acute interstitial pneumonia
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Nephrotic range proteinuria 2/2 minimal change
disease
All cultures and microscopic analysis negative
Diagnosis – Hamman Rich Syndrome
Hamman-Rich Syndrome
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Acute interstitial pneumonia
Described by Hamman and Rich in 1934
Rare and fulminant form of rapidly fibrosing
lung disease (idiopathic DAD)
Occurs in previously healthy individuals
without a history of lung disease, presents
within days to weeks of onset of symptoms
Unknown mechanism of the damage to the
pulmonary endothelium and epithelium
Hamman-Rich Syndrome
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Onset usually abrupt, prodromal illness lasts
7 to 14 days
Most common clinical signs and symptoms
are fever, cough, and shortness of breath
Not associated with cigarette smoking
Most patients are over the age of 40 years,
with a mean age of 50 to 55 years
Treatment
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Prognosis is poor, mortality rate >60%
at initial presentation.
Most patients die within 6 months
Treatment is supportive, attempt to
identify any possible cause
References
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Mandell’s Principles and Practices of Infectious Disease, 6th Ed.
Hamman, L, Rich, AR. Fulminating diffuse interstitial fibrosis of the lungs.
Trans Am Clin Climatol Assoc 1935; 51:154.
Vourlekis, JS. Acute interstitial pneumonia. Clin Chest Med 2004; 25:739.
Katzenstein, ALA, Myers, JL, Mazur, MT. Acute interstitial pneumonia. A
clinicopathologic, ultrastructural, and cell kinetic study. Am J Surg Pathol
1986; 10:256.
Bouros, D, Nicholson, AC, Polychronopoulos, V, du Bois, RM. Acute interstitial
pneumonia. Eur Respir J 2000; 15:412.6.Vourlekis, JS, Brown, KK, Cool, CD,
et al. Acute interstitial pneumonitis. Case series and review of the literature.
Medicine (Baltimore) 2000; 79:369.
Fulmer, JD, Katzenstein, ALA. The interstitial lung diseases. In: Pulmonary and
Critical Care Medicine, Bone, RC (Ed), Mosby Year Book, St. Louis; 1993,
M1.8.Olson, J, Colby, TV, Elliott, CG. Hamman-Rich syndrome revisited. Mayo
Clin Proc 1990; 65:1538.
Primack, SL, Hartman, TE, Ikezoe, J, et al. Acute interstitial pneumonia:
Radiographic and CT findings in nine patients. Radiology 1993; 188:817.
Johkoh, T, Muller, NL, Taniguchi, H, et al. Acute interstitial pneumonia: Thinsection CT findings in 36 patients. Radiology 1999; 211:859.